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Molecular mechanisms of neutrophil dysfunction in
glycogen storage disease type Ib
by Hyun Sik Jun, David A. Weinstein, Young Mok Lee, Brian C. Mansfield, and
Janice Y. Chou
Blood
Volume 123(18):2843-2853
May 1, 2014
©2014 by American Society of Hematology
G6PT-deficient neutrophils of GSD-Ib patients with varying maturation states were
dysfunctional.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
Analysis of 2-DG uptake, the expression of GLUT1 and HK, and levels of intracellular G6P,
lactate, and ATP in G6PT-deficient neutrophils of GSD-Ib patients.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
Analysis of NADPH and the expression of NADPH oxidase in G6PT-deficient neutrophils of GSDIb patients.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
Analysis of levels of Hsp90, HIF-1α, and PPAR-γ in G6PT-deficient neutrophils and the effects of
PPAR-γ antagonist/agonist on neutrophil function.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
Inhibition of neutrophil function by PPAR-γ is mediated via HIF-1α signaling.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
Proposed mechanisms that underlie neutrophil dysfunction in GSD-Ib.
Hyun Sik Jun et al. Blood 2014;123:2843-2853
©2014 by American Society of Hematology
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